Long-Latency Auditory Evoked Potentials in Adults with Williams Syndrome.

Background:  Williams syndrome (WS) is a genetic, multisystemic, neurodevelopmental disorder, in which studies have demonstrated the presence of auditory deficits such as conductive and sensorineural hearing loss, cochlear weakness, and subclinical signs of hearing impairment. However, few studies have assessed this population's central auditory system.

Purpose:  To analyze long-latency auditory evoked potential (LLAEP) in adults with WS and compare them with those obtained from neurotypical individuals with no hearing complaints.

Peripheral Auditory Pathway and ABR Characterization in Adults with Williams Syndrome.

 Williams syndrome (WS) is a genetic disorder caused by a microdeletion in chromosome 7, affecting ∼ 28 genes. Studies have demonstrated conductive losses seemingly related to the absence of the gene and mild to profound sensorineural losses due to cochlear fragility.  To characterize and compare the peripheral auditory system and auditory brainstem response (ABR) of adults with WS and neurotypical adults matched by age and gender.